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Topics to be discussed:

-Cleidocranial dysplasia-Hemifacial hyperplasia-Segmental odontomaxillary dysplasia-Lingual salivary gland depression-Focal osteoporotic bone marrow

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Cleidocranial dysplasia*Autosomal dominant syndrome affecting bones and teethAffect both sexes equallyCan be inherited or as a result of sporadic mutation-Runx2 gene

CCD affect mainly skull, clavicle and dentition-Face appear small in contrast to cranium-Bridge of nose may be broad and depressed Hypertelorism -Aplasia/hypoplasia of clavicleClinical features:

*parietal +Frontal bossingUnderdeveloped maxillaExcessive mobility of shoulderAppear shorter than unaffected relative -Prolonged retention of 1-Delayed eruption of 2 -Often have unerupted supernumerary teeth-extraction of 1 doesnt adequately stimulate eruption of 2

Lack of development of parietal bone*-a light-bulb shape due to brachycephaly-delayed or failure of fontanelles-open skull sutureLateral skull filmPosteroanterior skull filmRadiographic featuresMaxillary micrognathia-underdeveloped maxilla and paranasal sinus

*Absence of clavicleOpen fontanelWormian (sutural) bones in the occipital regionChest radiographLateral radiograph

3D CT reconstruction with oblique orientation*Parietal bossingOpen metopic sutureFrontal bossing

*Lack of normal coronoid processMultiple unerupted supernumerary teethMostly anterior max. n PM regionProlonged retention of 1 dentition

*Multiple of unerupted teethAxial CT view

done by :--Family history-excessive mobility of movement-Examination of skull-Radiographic finding

*Diagnosis

other disease associated with supernumerary and multiple unerupted teeth*2)Pycnodysotosis1)Gardners syndromePresence of clavicleAbnormally dense brittle boneShort statueBone osteomaMultiple intestinal polypsDifferential Diagnosis

-removal of 1 + supernumerary teeth-removal of bone overlying 2 to expose crown when half of the root is formed-monitor for any distal molar or cyst-surgical treatment for esthetic reason-CT scan*Management

Hemifacial dysplasia

Hemifacial hypertrophy, hemi hyperplasia

A condition that half of face including max. (alone @ with mand) @ other part of body to grow to unusual proportionCause:-unknown,may associate with genetic disease (Beckwith-Weidemann syndrome) *

*-Usually begin at birth-Often occur with other abnormalities: mental deficiency,skin abnormalities,compensatory scoliosis,genitourinary tract anomalies and neoplasm e.g. Wilms tumor of kidney,adrenocorticol tumor,hepatoblastoma (Beckwith-Weidemann syndrome)-F=M affectedDentition-Unilateral enlargement-accelerated development-premature loss of 1-enlarged tongue and alveolar one in affected sideclinical feature

If not detected during birth, it may become apparent during growth*-dysmorphic faceleft hemi facial hypertrophy ear lobe crease

*Radiographic feature :Rapid enlargement on right side of maxilla only-accelerated dental development for this 5-year-old patient

*Enlargement of bones include mand.maxilla,zygoma,frontal and temporal boneEnlargement of maxillary canine,1st PMCT axial image3D CT scan

*1)Hemifacial hypoplasia of the opposite side 2)Arteriovernous aneurysm3) Hemangioma 4) Congenital lymphadema5)Severe condylar hyperplasia that may involve half of mandible Differential Diagnosis

*D/D for case that limited to one side of maxillary1)Monostatic fibrous dysplasia--A rare bone disorder characterized by benign bone growths which can cause very painful swellings and bone deformities and makes bone prone to fractures.

2)Segmental odontomaxillary dysplasia

*-no significant case reported with long term follow-up , hence no definitive recommended treatment -generally those with suspected HH should be referred to a medical geneticist for diagnosis and early detection of genetic syndrome associatedManagement

Segmental odontomaxillary dysplasia-Hemimaxillofacial dysplasiaA developmental abnormality of unknown etiology that affect posterior alveolar process of one side of maxilla including teeth and attached gingivaDetected most in Childhood

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*--fullness of the right upper lip due to enlargement of the alveolar process.delayed eruption of teeth on the affected side Clinical features:-Always unilateral enlargement of alveolar process, gingiva and teeth-Frequently missing teeth (mostly PM)-Some teeth may unerupted at the affected side-unilateral hypertrichosis + mild facial enlargement some caseshypertrichosisfacial enlargementIntra-oral mirror image

*-a radiodensity that reduced the size of the right maxillary sinus- Both PM in the affected hemimaxilla were present-Maxilary sinus does not pneumatize the alveolar process-large left max.deciduos molars-lack of formation of bicuspids-delayed eruption of first molar -dense bone pattern of left maxillary alveolar process

*-Coarse trabecular pattern of right maxillary alveolar process-delayed eruption of maxillary right 1st PM and molars

*D/D :

1) Segmental hemifacial hyperplasia not associated with coarse vertically orieted trabeculae 2)Monostatic fibrous dysplasia-not associated with missing teeth3) Regional odontodysplasia -the teeth appear more radiolucent than normal, so described as "ghost teeth"

Lingual salivary depression*AsymptomaticIncidently finding

-lingual mandibular bone depression,developmental salivary gland defect, stafnes defect,stafnes bone cyst,static bone activity,latent bone cyst

-A group of concavities in lingual surface of mandible where depression is lined with an intact outer complex

Common location-within submandibular gland fossa-often close to inferior border of mandibular

*Sharpely defined radiolucencies beneath the mandibular canal in region of submandibular gland fossaThe defect can erode the inferior border of mandible

*Anterior variant within sublingual gland fossaUnusual variant with superior position above ID canalWhen defect is related to sublingual gland and appear above the canal,D/D could be odontogenic lesion

CT scan *

-well defined defect-Defect extending from mesial surface of the mandible-radiolucent tissue within the defectAxial bone window Axial soft tissue window3D reformatted CT image

*Differential Diagnosis :Appearance + location of radiographic image of the dfect are characteristic and easily identified

Epicenter of Odontogenic lesion is located above inf.alveolar canal

When defect is related to sublingual gland and appear above the canal,D/D could be odontogenic lesion

*Management recognition of lesion should preclude any treatment @ surgical exploration @ need for advancing image e.g CT

Defect may increase with time

Destruction of well defined cortex of defect may indicate neoplasm

Focal osteoporatic bone marrowMarrow spaceA radiologic term to indicate presence of radiolucent defects within the cancellous portion of jawHistologic exam-> normal area of hematopoitec or fatty marrowEtiology-unknown but is belief to be due to a) bone marrow hyperplasia b) persistent embryologic marrow remnant c) site of abnormal healing after extraction , trauma or local inflammation *

AsymptomaticIncidental radiograph findingMore common in middle aged-womenIt is consider as variation of normal anatomy*Clinical features:

*1)Internal aspect is Seen as a radiolucency2)Radiolucent due to few internal trabeculae present3)Periphery vary from well defined to ill defined Radiograph features

*Lesion located in furcation area of mandibulan 1st molar 4)Yet,PDL and lamina dura are intact

Could have same appearance-Simple bone cyst no bone reaction at periphery of it-Early inflammatory lesion with not yet stimulated a visible osteoblastic processIf occur in furcation region @ apex of tooth suspect inflammatory lesion*DIFFERENTIAL DIAGNOSIS

No treatment requiredIf in doubt , prescribe longitudinal study with films at 3-months interval -the bone marrow should not increase in size*Management

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Monostatic fibrous dysplasia-Fibrous dysplasia: poorly defined radiopaque lesion of the right maxilla.**